Primary Biliary Cirrhosis (PBC) is an autoimmune disease of the liver, which results in progressive destruction of the small bile ducts within the liver. It is most often seen in middle-aged women. It is a chronic, progressive disease, which eventually leads to cirrhosis and liver failure. It is often associated with other autoimmune disorders, such as thyroiditis.
Patients with PBC may experience itching, yellowing of the eyes and skin, fatigue, and collections of cholesterol in the skin around the eyes, known as xanthelasma. Ultimately, cirrhosis of the liver will ensue and may result in confusion, fluid retention and gastrointestinal bleeding.
The diagnosis of PBC is typically made when enzymes in the blood are elevated. A specific antibody known as AMA or Anti-Mitochondrial Antibody is elevated in most patients. The diagnosis is confirmed by performing a liver biopsy.
Ursodeoxycholic Acid is the most frequently used medication to treat this disease. It has been shown to improve liver blood tests and to slow the progression of the disease. Various medications can be used to treat symptoms, such as itching.